Systemic Autoimmune Diseases Flashcards
What are the major CT disease?
SLE Scleroderma Sjogren's Syndrome Auto-immune myositis Mixed CT disease e.g. Raynauds, soft tissue swelling
What are the major Systmic Vasculitides
GCA
GPA (Wegener’s)
Microscopic Polyangiitis
Churg-Strauss
Who gets SLE?
Women mostly
15-50yrs
More common in afro-caribbeans > Asians > caucasian
What criteria are needed to diagnose SLE?
4 or more of:
- Malar Rash
- Discoid rash
- Photosensitivity
- Oral ulcers
- Arthritis (at least 2 joints)
- Serositis (pleurisy or pericarditis)
- Renal (proteinuria or cellular casts in urine)
- Neuro (seizures or psychosis)
- Haemotology (Low WCC, platelets, lymphocytes or haemolytic anaemia)
- Immunological
- ANA: autoimmune antibodies
What is included in the “immunological” criteria for SLE?
Anti ds-DNA Abs Anti-SM Abs Anti Cardiolipin Abs Lupus Anticoagulant Low complement
Other than the primary criteria, what other symptoms/signs can come from SLE?
Alopecia Fever Depression Vasculitis, purpura & Urticaria Pleural effusion & Pulm fibrosis Raynaud's Aseptic necrosis of hip Myopathy Abdo Pain Aortic Valve Lesions Cranial nerve lesions, peripheral neuropathy and ataxia
Who gets Scleroderma?
Mostly female
Onset 30-50yrs
What are the types of scleroderma?
Localised (aka Morphea OR Linear) : only the skin is affected
Generalised (limited or Diffuse): internal organs are also affected.
What does localised scleroderma look like?
localised patches of hardened skin that are smooth and shiny.
Usually on the trunk
Painless
What does limited SCleroderma look like?
Thickening of skin and colour change in face and extremities along with Raynaud’s
CAn progress to affect gut –> heartburn & difficulty swallowing
What do you see in diffuse scleroderma?
Affects the whole body, with potential to hit the heart, lungs and kidneys
Expect fatigue, joint pain & stiffness
What is the general presentation of Scleroderma?
hardening of the skin swelling of the hands and feet joint pain stiffness Raynauds' Syndrome.
All due to excess collagen
What are the major complications of scleroderma?
Limited –> Pulm Hypertension
Diffuse –> Pulm fibrosis, renal crisis and small bowel bacterial overgrowth
Who gets Sjogren’s Syndrome?
Mostly women
40-50yrs
What’s the classic presentation of Sjogren’s Syndrome?
Dry eyes & mouth
Parotid Enlargement
~Systemic upset incl fever, fatigue, myalgia &arthralgia
Complications of Sjogren’s Syndrome?
Lymphoma Neuropathies (Cranial nerve, peripheral, fits etc) Purpura Interstitial Lung Disease Renal Tubular Acidosis
Auto-immune myositis is very rare. how does it present?
Symmetrical diffuse proximal muscle weakness.
Can be polymyositis or dermatomyositis
Dermatomyositis can come with Gottron’s papules (hands) and heliotrope rash
(face)
GCA affects the large arteries, what do you need to diagnose it?
3+ of:
- Age >50yrs at onset
- New headache
- Temporary art tenderness
- ESR >50
- Abnormal temporal biopsy
Which conditions are Anca associated Vasculitis?
Wegeners (GPA = granulomatosis with polyangiitis
Micrsocopic Polyangiitis
Eosinophilic Granulomatosis with polyangiitis
What happens in GPA?
Necrotising granulomatous INFLAMMATION
Occurs mainly in Upper & Lower resp tract in small and medium vessels along with necrotising glomerulonephritis
What’s different about MPA from GPA?
MPA doesn’t include immune deposites or granulomatous inflammation
Instead you get necrotising VASCULITIS of small vessels and necrotising glomerulonephritis. Sometimes with pulmonary capillaritis
What is Churg Strauss?
Eosinophilic Granulomatosis with Polyangiitis (EGPA)
Eosinophil rich necrotising granulomatous INFLAMMATION of small to med vessles
Mostly resp tract
Ass with asthma and eosinophilia
AAVs can lead to what kind of complications?
Hearing loss Renal failure Lung damage Malignancy Nerve damage
What ANA profiles are targeted in SLE?
dsDNA
Ro
Sm
What ANA profiles are targeted in Scleroderma?
Scl-70
Centromere
What ANA profiles are targeted in polymyositis?
Jo-1
What ANA profiles are targeted in Sjogren’s disease?
Ro
La
What other tests are relevant to these conditions?
FBC U&Es + Urinalysis CXR ECG ESR/CRP aPTT
Renal biopsy for nephritis
What are the stages of Lupus Nephritis?
1 - minimal mesangial 2 - mesangial proliferative 3 - focal 4 - diffuse 5 - membranous 6 - advanced sclerosing
How do we treat these conditions?
Mild - Hydroxychloroquine
Mod - Azathioprine, MTX or Mycophenolate
Severe - Cyclophosphomide or Rituximab
CCS can be used an adjunct or for specific symptoms but are not general 1st line treatments
Which antibodies are tested for in SLE patients who are pregnant and why?
Anti-Ro
Anti-La
They are both indicative of a high risk of neonatal Lupus
Try using this mnemonic to remember all the criteria for SLE: Dopamine Rash
Discoid Rash Oral Ulcer Photosensitvity Arthritis Malar Rash Immunological (Anti-sm, anti-dsDNA, Anti-phospholipid, anti-ro ; anti-la) Neuro symptoms (Psychosis, seizure, personality) ESR (raised)
Renal (Proteinuria or cellular casts)
ANA
Serositis (Pleurisy &Pericarditis)
Haematology (haemolytic anaemia, thrombocytopenia, leucopenia)
Pathogenesis of SLE?
Autoantibodies are formed against a subtype of autoantigens (ANA). This leads to the development of immune complexes. The inability to clear these complexes leads to hosts immune response.
